Publications by authors named "S M Siboni"

Type 3 von Willebrand disease (VWD), the most severe form of VWD, is an inherited recessive bleeding disorder caused by the complete deficiency of von Willebrand factor (VWF). The reported prevalence is 1 per million but varies worldwide according to the frequency of consanguineous marriages. The clinical phenotype is characterized not only by mucocutaneous bleedings, but also by hemarthroses and muscle hematoma, as in patients with moderate hemophilia.

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Article Synopsis
  • Early diagnosis of joint damage in haemophilia is crucial to prevent further complications and enhance patient management, with the HJHS and HEAD-US scores being primary evaluation tools.
  • This study aims to compare the effectiveness of HJHS and HEAD-US scores by two independent experts to assess joint health in patients with haemophilia.
  • Results show a strong correlation between the two methods, particularly highlighting the ultrasound's ability to detect mild synovitis even when physical examination shows no swelling, underscoring the importance of using both assessments in clinical practice.
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Objective: A definitive diagnosis of gastroesophageal reflux disease (GERD) depends on endoscopic and/or pH-study criteria. However, high resolution manometry (HRM) can identify factors predicting GERD, such as ineffective esophageal motility (IEM), esophago-gastric junction contractile integral (EGJ-CI), evaluating esophagogastric junction (EGJ) type and straight leg raise (SLR) maneuver response. We aimed to build and externally validate a manometric score (Milan Score) to stratify the risk and severity of the disease in patients undergoing HRM for suspected GERD.

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